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What is the neurological disease PSP?

DOCTOR OF THE MONTH - Sep 2013
Sep 2013
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Question
Are you familiar with the neurological disease PSP?
Posted Sat, 16 Aug 2014 in Brain and Spine
 
 
Answered by Dr. Het Ram Bishnoi 1 hour later
Brief Answer:
Progressive Supranuclear Palsy

Detailed Answer:
Hello XXXX,
I am writing in detail about PSP ( Progressive Supranuclear Palsy).
This is a sporadic NEURODEGERATIVE disorder of UNKNOWN etiology associated with tau pathology.
It usually presents in the 7th to 8th decades and progress faster than Parkinsonism"s disease .
Risks factors are :
Head trauma
Vascular disease
Dietary exposure to benzyle-tetrahydroisoquinolines (TIQ, reticulin)
Beta carbolines
PSP is characterized by :
Akinetic rigid parkinsonism
Dizziness
u\Unsteadiness
Slowness
Falls,
Pseudobulbar dysarthria
TREMOR IS DISTINCTLY UNCOMMON.
First of all occurs Supranuclear eye movements abnormalities affecting downgaze .After this there occurs limitation of upward and horizontal movement of eyes.
Because the vestibular ocular reflex abnormal while Bell's reflex (elevation and abduction of eyes on attempted lid closure ) intact ,these abnormalities termed as SUPRANUCLEAR , that's why known as PROGRESSIVE SUPRANUCLEAR PALSY ( PSP).
Symptoms & signs:
Prominent stare & furrowed brow
Axial & proximal distal limb rigidity ( as in your patient's case)
Dystonia
Cerebellar signs as incordination of movement and ataxia .
Frontal type cognitive dysfunction.
Dementia
Get in brain M R I for confirmation ( find mid brain atrophy)
PET study shows symmentric frontal & striatal hypometabolism.
Now treatment ,although some response may occur to levodopa especially early in the course but treatment is NOT EFFECTIVE.
The diagnosis made only on CLINIC grounds.
There is loss of dopamine & dopamine receptors due to intrinsic striatal damage.This is thought to be the poor response to therapy .Prognosis is poor.
Hope this will help you .Good luck .
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